Richter's hernia of the splenic flexure: computed tomography appearances.

A case of a clinically occult Richter's hernia of the splenic flexure, through an anterior abdominal wall defect, is described. In view of the initial absence of bowel obstruction with a partial or Richter's hernia, and in the absence of physical findings, the importance of computed tomography (CT) in demonstrating abdominal wall lesions is highlighted.

Multiple endocrine neoplasm, type 1. Gastrinomas, pancreatic neoplasms, microcarcinoids, the Zollinger-Ellison syndrome, lymph nodes, and hepatic metastases.

OBJECTIVE: We reviewed the age of presentation, malignant potential, and outcome of gastrinomas and pancreatic tumors in patients with multiple endocrine neoplasm, type 1. DESIGN: Screening of one very large and one smaller, possibly related family on an island, including serum gastrin estimations and, when indicated, pancreatic ultrasound. SETTING AND PATIENTS: Over 2000 family members and their family physicians were advised on screening procedures. INTERVENTION: Data were collected and reviewed retrospectively and prospectively for all medical records, investigations, surgical procedures, and available tissue samples. OUTCOME MEASUREMENTS: Criteria for diagnosis were established for radiological, biochemical, and histological studies. RESULTS: Sixty-two patients had evidence of gastrinoma or pancreatic neoplasm. In 19 patients the diagnosis was based on demonstration of a tumor. In 21 patients the diagnosis was based on elevated serum gastrin concentration in the absence of demonstrable tumor. None of these patients required gastric surgery if they first underwent parathyroidectomy. In 18 patients the diagnosis was based on the combination of demonstrated pancreatic tumor plus elevated glucagon (two patients), gastrin (11 patients), or insulin (five patients) concentration. Peptic ulcer was difficult to control in seven of the 11 patients with elevated gastrin concentrations plus demonstrated tumor. Four patients had liver metastases that appeared to be secondary to the pancreatic gastrinoma. In patients with insulinomas, the first symptoms occurred before age 20 years. Elevated serum gastrin concentrations were not seen before age 24 years and were observed to occur for the first time in two patients after age 50 years.

Cause of death in multiple endocrine neoplasia type 1.

OBJECTIVE: Little data are available on the natural history of untreated multiple endocrine neoplasia type 1 (MEN-1). These data are essential in deciding treatment that may carry significant morbidity. We determined the causes of death in a large MEN-1 kindred with data available over a period of 130 years. Most cases were unrecognized as MEN-1 at the time of patient's death. DESIGN: Retrospective study of recorded medical data from 1861 to 1991. PATIENTS: One hundred fifty-nine deaths occurred, of which 46 were in individuals classified as "highly probable" of having MEN-1. RESULTS: Of 46 deaths in those classified as "highly probable" of having MEN-1, 20 (43.5%) died of a recognized complication of MEN-1 (12 of malignant neoplasms, six of renal calculi, and two of peptic ulcer). If accidental deaths are excluded, 50% of the deaths in patients with MEN-1 were the result of MEN-1, and the mean age of death (50.9 years)was significantly younger than that of other family members. CONCLUSIONS: It is concluded that MEN-1 leads to premature death, and that neoplasia rather than peptic ulcer disease is the main cause of death. Deaths from pituitary tumor or malignant endocrine tumors within the thorax were just as common or more common than deaths from pancreatic malignant neoplasms.

Three-dimensional assessment of surgical excision margins in skin tumor resection biopsies.

A new technique for the assessment of adequacy of surgical margins of excision in skin tumor resection biopsies is described. The technique essentially consists of staining the whole fresh or fixed biopsy tissue in eosin followed by Mayer's hematoxylin. Following staining, the epidermal perimeter of the skin ellipse and the deep surgical margin can be assessed using a stereo-microscope. Should areas suspicious of inadequate excision be identified these may be confirmed by directed frozen or paraffin section.

Primary rhabdomyosarcoma of the adult liver.

A 53-year-old male Caucasian presented with a massive liver tumor composed entirely of spindle-shaped cells showing light-microscopic and ultrastructural evidence of rhabdomyoblastic differentiation. No epithelial or other sarcomatous elements were included. Detailed postmortem examination failed to reveal evidence of metastatic tumor or an alternative primary site. There have been only four previous cases of primary rhabdomyosarcoma of the adult liver, all occurring in Japanese men.

Limbic encephalitis associated with malignant thymoma.

A case of limbic encephalitis associated with recurrent malignant thymoma in a 41-year-old male is described. The patient presented with confusion, loss of memory, hallucinations, abnormal behaviour, tachycardia and profuse sweating. Investigations were unrewarding and the patient's clinical state deteriorated until his death 1 month after presentation. The diagnosis was made at autopsy when bilateral extensive neuronal loss with reactive gliosis, confined to the medial temporal cortex and Ammon's horn, was revealed.

Quantitative assessment of Langerhans cells in actinic keratosis, Bowen's disease, keratoacanthoma, squamous cell carcinoma and basal cell carcinoma.

The quantitative distribution of Langerhans cells (LC) was studied in a range of pre-neoplastic, in-situ and invasive neoplastic skin lesions using an antibody to S100 protein and the indirect immunoperoxidase technique. LC numbers were increased within the lesions of actinic keratosis, Bowen's disease, keratoacanthoma, squamous cell carcinoma and basal cell carcinoma. In all lesions except actinic keratosis the LC density was also significantly increased in the adjacent non-neoplastic epithelium. The increased LC density in neoplastic epithelium suggests either that LC are being retained within the abnormal epithelium for longer periods of time than normal or that increased numbers of LC are being actively attracted by factors produced by the neoplastic epithelium. While reduction of intraepithelial LC density may allow the initiation of neoplasia the increased density observed in this study suggests that at later stages of tumour growth LC may have a functional role in the host response to cutaneous neoplasia.

Quantitative assessment of Langerhans' cells in human cervical intraepithelial neoplasia and wart virus infection.

Langerhans' cell density was assessed quantitatively in cervical wart virus infection (cervical condyloma), cervical intraepithelial neoplasia, and koilocytic dysplasia with use of an antibody to S100 protein and an indirect immunoperoxidase technique. When compared with normal ectocervix, Langerhans' cell density was significantly decreased in cervical wart virus infection and significantly increased in cervical intraepithelial neoplasia. In koilocytic dysplasia, intermediate Langerhans' cell densities were obtained. In addition to being increased within the lesions of cervical intraepithelial neoplasia, Langerhans' cell density was increased in the adjacent normal ectocervix. Human papillomavirus, by reducing intraepithelial Langerhans' cell density, may decrease local immune surveillance and thus have a promoter effect in the development of cervical cancer. Following the development of cervical intraepithelial neoplasia the increase in intraepithelial Langerhans' cell density suggests a specific immune response directed against neoantigens associated with malignant transformation. If a permissive wart virus infection persists after transformation to cervical intraepithelial neoplasia (koilocytic dysplasia), continued depletion of Langerhans' cells results in intermediate densities.

New methodology for assessment of the Langerhans cell network.

A new procedure is described for staining Langerhans cells (LCs) based on the ability of anti-S-100 antibody to stain both epidermal LCs and melanocytes, while L-Dopa stains only melanocytes. This procedure can be used on paraffin-embedded skin sections and is therefore advantageous for examination of pathological skin specimens. In order to determine how best to quantitate LCs in skin sections the distribution of LCs has been investigated using an improved method for preparation of epidermal sheets from mouse skin. Epidermal LCs stained for their surface membrane-bound enzyme adenosine triphosphatase were observed to link with each other via their dendrites, forming a single cell layer which undulates throughout the epidermis. It is therefore proposed that LCs in skin sections should be enumerated per unit length, after identification in paraffin-embedded sections double stained with anti-S-100 antibody and L-Dopa.

Basal lamina redevelopment in tumours metastatic to brain:an immunoperoxidase study using an antibody to type-IV collagen.

The basal lamina in a variety of metastatic tumours in brain was assessed with an antibody to type-IV collagen and the indirect immunoperoxidase technique. The antibody was raised in rabbits against type-IV collagen isolated from human placental tissue. Basal lamina redevelopment was demonstrated around individual melanoma cells, between melanoma cells and cerebral parenchyma, and at the tumour-stroma interface in both metastatic melanoma and metastatic carcinoma. At the periphery of metastatic carcinomatous deposits, no basal lamina was observed between tumour cells and the adjacent cerebral parenchyma. Basal lamina staining other than that of cerebral vessels was absent in deposits of poorly differentiated tumours which were unaccompanied by the development of a tumour stroma. It is concluded that metastatic tumours retain the ability to produce basal lamina and, in the case of metastatic epithelial tumours, the redevelopment of basal lamina is dependent on interaction with mesenchymal tissue. The stromal dependency of basal lamina formation by metastatic epithelial tumours suggests the reactivation of a control mechanism acting in normal tissue. Although basal lamina formation in metastatic melanoma can occur in the absence of mesenchymal tissue, there may be some interaction between tumour cells and stroma in the redevelopment of basal lamina at the tumour-stroma interface.

Characterization of retraction spaces in basal cell carcinoma using an antibody to type IV collagen.

'Retraction spaces' observed in 18 of 30 basal cell carcinomas were readily classified into two distinct types using the PAP immunoperoxidase technique and an antibody to human type IV collagen, raised in rabbits. In processing artefacts, true retraction spaces were observed between the epithelial cells and the basal lamina, while in areas of stromal mucinous change, accumulation of mucinous material resulted in clear spaces separating the normal stroma from the overlying basal lamina.

The basal lamina in basal cell carcinoma, Bowen's disease, squamous cell carcinoma and keratoacanthoma: an immunoperoxidase study using an antibody to type IV collagen.

The basal lamina in a variety of skin tumours was assessed with an antibody to type IV collagen and the peroxidase-antiperoxidase (PAP) technique. The antibody was raised in rabbits against type IV collagen isolated from human placental tissue. The basal lamina in Bowen's disease was essentially intact while squamous cell and basal cell carcinomas showed focal loss in areas of tumour cell invasion. However, both tumours showed preservation of basal lamina around the majority of projections and nests of tumour within the dermis. Many keratoacanthomas showed extensive loss of the basal lamina. This loss appears associated with advanced keratinization at the base of the lesion and may represent an involutional change possibly secondary to inflammation. It is concluded that epidermal tumour cells following local invasion may cease migration and produce a distinct continuous basal lamina similar to that of the normal dermo-epidermal junction. Loss of basal lamina appears restricted to foci of ongoing tumour invasion.